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1.  Long-Term Follow-Up Result of Hydroxyurea Chemotherapy for Recurrent Meningiomas 
Objective
Meningiomas represent 18-20% of all intracranial tumors and have a 20-50% 10-year recurrence rate, despite aggressive surgery and irradiation. Hydroxyurea, an inhibitor of ribonucleotide reductase, is known to inhibit meningioma cells by induction of apoptosis. We report the long-term follow-up result of hydroxyurea therapy in the patients with recurrent meningiomas.
Methods
Thirteen patients with recurrent WHO grade I or II meningioma were treated with hydroxyurea (1000 mg/m2/day orally divided twice per day) from June 1998 to February 2012. Nine female and 4 male, ranging in age from 32 to 83 years (median age 61.7 years), were included. Follow-up assessment included physical examination, computed tomography, and magnetic resonance imaging (MRI). Standard neuro-oncological response criteria (Macdonald criteria) were used to evaluate the follow-up MRI scans. The treatment was continued until there was objective disease progression or onset of unmanageable toxicity.
Results
Ten of the 13 patients (76.9%) showed stable disease after treatment, with time to progression ranging from 8 to 128 months (median 72.4 months; 6 patients still accruing time). However, there was no complete response or partial response in any patients. Three patients had progressive disease after 88, 89, 36 months, respectively. There was no severe (Grade III-IV) blood systemic disorders and no episodes of non-hematological side effects.
Conclusion
This study showed that hydroxyurea is a modestly active agent against recurrent meningiomas and can induce long-term stabilization of disease in some patients. We think that hydroxyurea treatment is well tolerated and convenient, and could be considered as an alternative treatment option in patients with recurrent meningiomas prior to reoperation or radiotherapy.
doi:10.3340/jkns.2012.52.6.517
PMCID: PMC3550418  PMID: 23346322
Meningioma; Chemotherapy; Hydroxyurea; Recurrence
2.  Intradural Extramedullary and Subcutaneous Tumors in Neonate : Atypical Myxoid Spindle Cell Neoplasm 
Tumors of the central nervous system are common in the pediatric population and constitute the second most prevalent tumor type in children. Within this group, spinal cord tumors are relatively rare and account for 1 to 10% of all pediatric central nervous system tumors. We describe a very rare case of an intradural extramedullary spinal cord tumor with a subcutaneous mass and discuss its clinical presentation, pathogenesis, and treatment. A male infant was delivered normally, with uneventful development. At 16 days post-delivery, his family took him to a pediatrician because of a mass on his upper back. Magnetic resonance imaging of the thoracic spine revealed a well-demarcated soft-tissue mass with central cystic change or necrosis at the subcutaneous layer of the posterior back (T2-7 level). Another mass was found with a fat component at the spinal canal of the T1-3 level, which was intradural extramedullary space. After six weeks, the spinal cord tumor and subcutaneous mass were grossly total resected; pathologic findings indicated an atypical myxoid spindle cell neoplasm, possibly nerve sheath in type. The final diagnosis of the mass was an atypical myxoid spindle cell neoplasm. The postoperative course was uneventful, and the patient was discharged after nine days without any neurological deficit. We report a rare case of an intradural extramedullary spinal tumor with subcutaneous mass in a neonate. It is necessary to monitor the patient's status by examining consecutive radiologic images, and the symptoms and neurological changes should be observed strictly during long-term follow-up.
doi:10.3340/jkns.2012.52.4.417
PMCID: PMC3488656  PMID: 23133736
Intradural extramedullary spinal cord neoplasm; Neonate; Congenital
3.  Neurotization from Two Medial Pectoral Nerves to Musculocutaneous Nerve in a Pediatric Brachial Plexus Injury 
Traumatic brachial plexus injuries can be devastating, causing partial to total denervation of the muscles of the upper extremities. Surgical reconstruction can restore motor and/or sensory function following nerve injuries. Direct nerve-to-nerve transfers can provide a closer nerve source to the target muscle, thereby enhancing the quality and rate of recovery. Restoration of elbow flexion is the primary goal for patients with brachial plexus injuries. A 4-year-old right-hand-dominant male sustained a fracture of the left scapula in a car accident. He was treated conservatively. After the accident, he presented with motor weakness of the left upper extremity. Shoulder abduction was grade 3 and elbow flexor was grade 0. Hand function was intact. Nerve conduction studies and an electromyogram were performed, which revealed left lateral and posterior cord brachial plexopathy with axonotmesis. He was admitted to Rehabilitation Medicine and treated. However, marked neurological dysfunction in the left upper extremity was still observed. Six months after trauma, under general anesthesia with the patient in the supine position, the brachial plexus was explored through infraclavicular and supraclavicular incisions. Each terminal branch was confirmed by electrophysiology. Avulsion of the C5 roots and absence of usable stump proximally were confirmed intraoperatively. Under a microscope, neurotization from the musculocutaneous nerve to two medial pectoral nerves was performed with nylon 8-0. Physical treatment and electrostimulation started 2 weeks postoperatively. At a 3-month postoperative visit, evidence of reinnervation of the elbow flexors was observed. At his last follow-up, 2 years following trauma, the patient had recovered Medical Research Council (MRC) grade 4+ elbow flexors. We propose that neurotization from medial pectoral nerves to musculocutaneous nerve can be used successfully to restore elbow flexion in patients with brachial plexus injuries.
doi:10.3340/jkns.2012.52.3.267
PMCID: PMC3483334  PMID: 23115676
Brachial plexus; Neurotization; Pectoral nerve; Musculocutaneous nerve
4.  Subarachnoid Hemorrhage with Negative Baseline Digital Subtraction Angiography: Is Repeat Digital Subtraction Angiography Necessary? 
Introduction
Patients with negative initial digital subtraction angiography (DSA) are at significant risk for re-bleeding, which can lead to severe disability and death. The purpose of this study was to evaluate the necessity of repeat DSA in subgroups of patients with subarachnoid hemorrhage (SAH) with negative initial DSA.
Methods
A total of 904 spontaneous SAH patients were admitted to our department between May 2005 and May 2012. Twenty eight patients were selected for inclusion in this study because repeated DSA performed due to the etiology of the SAH could not be demonstrated on the initial DSA. According to the SAH pattern on initial computed tomography scans, patients were divided into perimesencephalic nonaneurysmal SAH (PN-SAH) and non PN-SAH (NPN-SAH) groups. Repeat DSA was performed in all patients, and two of these patients underwent a third DSA.
Results
Of the 904 patients, 28 patients (3.1%) had no vascular abnormality on initial DSA. Sixteen PN-SAH patients underwent a repeat DSA; however, no aneurysms were found. In contrast, 12 patients with NPN-SAH underwent repeat DSA, with detection of two cerebral aneurysms. Overall, the false-negative rate of the initial DSA was 7.1% (2/28 patients). No significant differences in false-negative results on initial DSA were observed between the PN-SAH and NPN-SAH groups.
Conclusion
In the line with the results of the current study, we should be highly suspicious of patients with a nonaneurysmal SAH, especially those with a NPN-SAH pattern. In order to reduce the morbidity and mortality resulting from a misdiagnosis, repeat DSA is necessary, and exclusion of an aneurysm is important.
doi:10.7461/jcen.2012.14.3.210
PMCID: PMC3491216  PMID: 23210049
Subarachnoid hemorrhage; Digital subtraction angiography; Aneurysm

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